'Rainbow pattern': a dermoscopic sign of invasive melanoma.

BACKGROUND: The 'rainbow pattern' was initially described as a highly specific dermoscopic feature of Kaposi sarcoma. Since then, it has been reported in many benign and malignant cutaneous tumours, including a few malignant melanomas (MMs). AIM: To determine the frequency and presentation of this dermoscopic pattern in primary cutaneous MMs in comparison to other cutaneous tumours. METHODS: The presence of a rainbow pattern was evaluated in a sample of 1100 dermoscopic images of different melanocytic and nonmelanocytic cutaneous neoplasms. RESULTS: The rainbow pattern was observed in 23 of 245 (9.4%) MM and 44 of 855 (5.1%) non-MM neoplasms. MMs presenting this feature were generally thicker: 82.6% > 1 mm and 43.0% > 2 mm. Compared with non-MMs, rainbow pattern in MMs was more commonly focal (82.7% vs. 36.4% nonfocal, P = 0.001) and associated with > 2 dermoscopic structures associated with MM (100% vs. 9% with fewer, P = 0.001). CONCLUSION: The rainbow pattern is a dermoscopic sign that can occasionally be observed in invasive MMs. In MMs, this feature is usually associated with other dermoscopic criteria of MM and located in a focal and eccentric area, as opposed to a diffuse and isolated presentation in non-MM neoplasms.

Leiomiosarcoma cutáneo: características clínicas, histopatológicas y correlación pronóstica en 12 pacientes / Clinical and Histopathologic Findings of Cutaneous Leiomyosarcoma: Correlation with Prognosis in 12 Patients

INTRODUCCIÓN: El leiomiosarcoma de piel es una neoplasia maligna de estirpe muscular cuya baja incidencia dificulta el desarrollo de protocolos específicos de diagnóstico y manejo terapéutico. OBJETIVOS: Describir las características clínicas e histopatológicas de una serie de leiomiosarcomas cutáneos primarios y secundarios, junto con su correlación pronóstica. MATERIAL Y MÉTODOS: Se realizó un estudio retrospectivo, descriptivo y observacional. Se seleccionaron 17 casos de leiomiosarcoma cutáneo en 12 pacientes, diagnosticados entre el 1 de enero de 2000 y el 31 de diciembre de 2015. Se recogieron sus datos demográficos, características clínicas e histopatológicas, evolución y respuesta al tratamiento. RESULTADOS: Se reclutaron 5 varones y 7 mujeres, todos ellos mayores de 50 años al diagnóstico. Se recogieron 4 leiomiosarcomas dérmicos (4/17, 23%) en 4 pacientes, 2 leiomiosarcomas hipodérmicos (2/17, 11,5%) en 2 pacientes, y 11 metástasis cutáneas de leiomiosarcoma (11/17, 65%) en 6 pacientes. Las localizaciones más frecuentes fueron cuero cabelludo (7/17, 41%), miembros inferiores (3/17, 17%) y tronco (3/17, 17%). Durante el seguimiento, un 50% de leiomiosarcomas dérmicos recidivaron, un 50% de leiomiosarcomas hipodérmicos presentaron metástasis a distancia y 5/6 pacientes con metástasis cutáneas de leiomiosarcoma (83%) fallecieron a causa de su enfermedad. Limitaciones: Este estudio es una revisión retrospectiva de una serie de casos de tamaño limitado en un centro único. CONCLUSIONES: El leiomiosarcoma cutáneo es una neoplasia maligna poco frecuente. A la hora de adoptar una actitud diagnóstico-terapéutica en estos pacientes debemos tener en cuenta la marcada heterogeneidad pronóstica entre sus diferentes subtipos

INTRODUCTION: Cutaneous leiomyosarcoma is a malignant neoplasm derived from smooth muscle cells. Its low incidence hampers the development of specific protocols for diagnosis and treatment. OBJECTIVES: To describe the clinical and histopathologic characteristics of a series of primary and secondary cutaneous leiomyosarcomas and to determine how these characteristics correlate with prognosis. MATERIAL AND METHODS: We performed an observational, descriptive, retrospective study based on 17 cutaneous leiomyosarcomas in 12 patients diagnosed between January 1, 2000 and December 31, 2015. We recorded demographic data, clinical and histopathologic characteristics, outcome, and response to treatment. RESULTS: We included 5 men and 7 women, all aged more than 50 years at diagnosis. There were 4 cutaneous leiomyosarcomas (23%) in 4 patients, 2 subcutaneous leiomyosarcomas (11.5%) in 2 patients, and 11 skin metastases of leiomyosarcoma (65%) in 6 patients. The most frequently affected sites were the scalp (41%), lower limbs (17%), and trunk (17%). During follow-up, 50% of the cutaneous leiomyosarcomas recurred, 50% of the subcutaneous leiomyosarcomas presented distant metastases, and 83% of the patients with skin metastases of leiomyosarcoma died of their disease. Limitations: Ours was a retrospective review of a small case series at a single center. CONCLUSIONS: Cutaneous leiomyosarcoma is an uncommon malignant neoplasm. Our approach to diagnosis and therapy must take into account the marked heterogeneity in the prognosis of the various subtypes

Clinical and Histopathologic Findings of Cutaneous Leiomyosarcoma: Correlation with Prognosis in 12 Patients. / Leiomiosarcoma cutáneo: características clínicas, histopatológicas y correlación pronóstica en 12 pacientes.

INTRODUCTION: Cutaneous leiomyosarcoma is a malignant neoplasm derived from smooth muscle cells. Its low incidence hampers the development of specific protocols for diagnosis and treatment. OBJECTIVES: To describe the clinical and histopathologic characteristics of a series of primary and secondary cutaneous leiomyosarcomas and to determine how these characteristics correlate with prognosis. MATERIAL AND METHODS: We performed an observational, descriptive, retrospective study based on 17 cutaneous leiomyosarcomas in 12 patients diagnosed between January 1, 2000 and December 31, 2015. We recorded demographic data, clinical and histopathologic characteristics, outcome, and response to treatment. RESULTS: We included 5 men and 7 women, all aged more than 50 years at diagnosis. There were 4 cutaneous leiomyosarcomas (23%) in 4 patients, 2 subcutaneous leiomyosarcomas (11.5%) in 2 patients, and 11 skin metastases of leiomyosarcoma (65%) in 6 patients. The most frequently affected sites were the scalp (41%), lower limbs (17%), and trunk (17%). During follow-up, 50% of the cutaneous leiomyosarcomas recurred, 50% of the subcutaneous leiomyosarcomas presented distant metastases, and 83% of the patients with skin metastases of leiomyosarcoma died of their disease. LIMITATIONS: Ours was a retrospective review of a small case series at a single center. CONCLUSIONS: Cutaneous leiomyosarcoma is an uncommon malignant neoplasm. Our approach to diagnosis and therapy must take into account the marked heterogeneity in the prognosis of the various subtypes.

Hiperqueratosis folicular filiforme en la cara / Filiform Follicular Hyperkeratosis on the Face

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Hemangiomas glomeruloides y síndrome POEMS / Glomeruloid hemangioma and POEMS syndrome

El síndrome POEMS es una manifestación paraneoplásica asociada a procesos hematológicos como el mieloma múltiple y la enfermedad de Castleman. El acrónimo engloba las manifestaciones clínicas más frecuentes (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin abnormalities). Dentro de las manifestaciones cutáneas, destaca por su especificidad la aparición de hemangiomas glomeruloides. Pese a no ser patognomónicos de la enfermedad, su aparición debe hacer sospechar la presencia del síndrome POEMS o su eventual desarrollo, ya que pueden aparecer años antes del desarrollo completo del síndrome. Presentamos 2 mujeres adultas con discrasias de células plasmáticas y aparición brusca de lesiones de aspecto vascular compatibles histológicamente con hemangiomas glomeruloides. Debemos reconocer la posible aparición de este tipo de tumores vasculares en los pacientes con síndrome POEMS para su diagnóstico precoz

POEMS syndrome is a paraneoplastic manifestation associated with hematopoietic disorders such as multiple myeloma and Castleman disease. POEMS is an acronym for the main clinical features of the syndrome, namely, Polyneuropathy, Organomegaly, Endocrinopathy, M protein, and Skin abnormalities. Glomeruloid hemangiomas are considered to be a specific clinical marker of POEMS syndrome. However, while they are not pathognomonic, their presence should raise suspicion of this syndrome or alert clinicians to its possible future development, as these lesions can appear years before the onset of the syndrome. We report the cases of 2 women with plasma cell dyscrasias and sudden onset of lesions with a vascular appearance and histologic findings consistent with glomeruloid hemangioma. Recognition of this vascular tumor is important for the early diagnosis of POEMS syndrome

Glomeruloid hemangioma and POEMS syndrome. / Hemangiomas glomeruloides y síndrome POEMS.

POEMS syndrome is a paraneoplastic manifestation associated with hematopoietic disorders such as multiple myeloma and Castleman disease. POEMS is an acronym for the main clinical features of the syndrome, namely, Polyneuropathy, Organomegaly, Endocrinopathy, M protein, and Skin abnormalities. Glomeruloid hemangiomas are considered to be a specific clinical marker of POEMS syndrome. However, while they are not pathognomonic, their presence should raise suspicion of this syndrome or alert clinicians to its possible future development, as these lesions can appear years before the onset of the syndrome. We report the cases of 2 women with plasma cell dyscrasias and sudden onset of lesions with a vascular appearance and histologic findings consistent with glomeruloid hemangioma. Recognition of this vascular tumor is important for the early diagnosis of POEMS syndrome.

Tricogerminoma: una neoplasia con diferenciación folicular y una morfología característica / Trichogerminoma: a neoplasm with follicular differentiation and a characteristic morphology

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Síndrome de descamación de la piel acral: presentación de un caso y revisión bibliográfica / Acral peeling skin syndrome: a case report and literature review

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Congenital cutaneous Langerhans cell histiocytosis and cutaneous mastocytoma in a child.

Langerhans cell histiocytosis and mastocytoma are clonal disorders of bone-marrow-derived cells, most commonly seen in the pediatric age. Infiltration of mast cells and Langerhans cells in the same lesion has been published before, but, to our knowledge, this is the first time that the occurrence of two mastocytomas and Langerhans cell histiocytosis is reported. It could be hypothesized that both clonal disorders of bone-marrow-derived cells could have a common origin.

Dermatitis granulomatosa como manifestación cutánea de trastornos hematológicos: primer caso asociado a policitemia vera y un nuevo caso asociado a mielodisplasia / Granulomatous Dermatitis as a Cutaneous Manifestation of Hematologic Disorders: The First Case Associated With Polycythemia Vera and a New Case Associated With Myelodysplasia

Las dermatitis de patrón granulomatoso se han relacionado con trastornos hematológicos, entre ellos los síndromes mielodisplásicos. En este artículo se describe el primer caso de dermatitis granulomatosa asociado a policitemia vera, en forma de grandes nódulos eritematosos simulando paniculitis, y el séptimo caso asociado a mielodisplasia, con placas eritematosas en cara y cuello que recordaban a una dermatosis neutrofílica. Consideramos de especial interés para el dermatólogo conocer esta dermatosis como manifestación no específica de diferentes trastornos hematológicos, y sugerimos la realización de un estudio complementario (hemograma) en el caso de que la clínica cutánea sea el comienzo. Del mismo modo, proponemos el término más amplio de dermatitis granulomatosas para denominarlas, puesto que, si bien las formas intersticiales son más numerosas en la literatura, también se han publicado casos más nodulares, y la importancia de su diagnóstico no radica en el subtipo sino en su relación con un trastorno subyacente

Granulomatous dermatitis has been associated with hematologic disorders, including the myelodysplastic syndromes. We describe the first case of granulomatous dermatitis associated with polycythemia vera, presenting as large erythematous nodules mimicking panniculitis. We also present the seventh case associated with myelodysplasia, with erythematous plaques on the face and neck, similar to a neutrophilic dermatosis. We consider it particularly interesting for dermatologists to be aware of this dermatosis as a nonspecific manifestation of various hematologic disorders. We suggest performing additional tests (complete blood count) to exclude the possibility that the skin manifestations are the initial sign of hematologic disease. Furthermore, we propose using the broader term, granulomatous dermatitis, to refer to these disorders as, although there are more reports of interstitial forms, cases with a more nodular presentation have also been published, and the importance of the diagnosis derives not from the subtype but from the relationship with an underlying disease

Granulomatous Dermatitis as a Cutaneous Manifestation of Hematologic Disorders: The First Case Associated With Polycythemia Vera and a New Case Associated With Myelodysplasia. / Dermatitis granulomatosa como manifestación cutánea de trastornos hematológicos: primer caso asociado a policitemia vera y un nuevo caso asociado a mielodisplasia.

Granulomatous dermatitis has been associated with hematologic disorders, including the myelodysplastic syndromes. We describe the first case of granulomatous dermatitis associated with polycythemia vera, presenting as large erythematous nodules mimicking panniculitis. We also present the seventh case associated with myelodysplasia, with erythematous plaques on the face and neck, similar to a neutrophilic dermatosis. We consider it particularly interesting for dermatologists to be aware of this dermatosis as a nonspecific manifestation of various hematologic disorders. We suggest performing additional tests (complete blood count) to exclude the possibility that the skin manifestations are the initial sign of hematologic disease. Furthermore, we propose using the broader term, granulomatous dermatitis, to refer to these disorders as, although there are more reports of interstitial forms, cases with a more nodular presentation have also been published, and the importance of the diagnosis derives not from the subtype but from the relationship with an underlying disease.

Pitiriasis liquenoide y varioliforme aguda / Pityriasis lichenoides et varioliformis acuta

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Lesiones nódulo-quísticas sobre placa de alopecia congénita / Nodular-Cystic Lesions on a Congenital Alopecic Plaque

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